CF

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  1. Epidemiology of CF
    • Most common life limiting disorder in Caucasians
    • Current life expectancy of ~36 years
  2. Pathophysiology of CF
    • Autosomal recessive
    • Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)
    • → Most common mutation: ΔF508
    • CFTR regulates Na+ and Cl- ion transport and salt homeostasis in sweat glands
    • → Mutated: sweat contains large amounts of salt and leads to a negatively charged lumen
    •     ¤ Water follows salt, mucous membranes become dry, no longer nice and stretchy mucous
    • → Results in organ damage
  3. Organ: Reproductive
    Abnormality:
    Result:
    • Organ: Reproductive
    • Abnormality:
    • → Obstruction in male reproductive tract
    • Result:
    • → Azospermia
    • → Infertility
    •     ¤ Harder to get pregnant
  4. Organ: Pancreas
    Abnormality:
    Result:
    • Organ: Pancreas
    • Abnormality:
    • → Lack of pancreatic enzymes
    • → Poor insulin function
    • Result:
    • → Poor absorption
    • → Malnutrition
    • → CF Diabetes
  5. Organ: Sweat glands
    Abnormality:
    Result:
    • Organ: Sweat glands
    • Abnormality:
    • → Unable to absorb sodium
    • Result:
    • → Hyponatremia
  6. Organ: Liver
    Abnormality:
    Result:
    • Organ: Liver
    • Abnormality:
    • → Cirrhosis
    • → Fatty infiltration
    • Result:
    • → Portal hypertension
    • → Esophageal varices
    •     ¤ Need liver transplant
  7. Organ: Intestines
    Abnormality:
    Result:
    • Organ: Intestines
    • Abnormality:
    • → Thick secretions
    • Result:
    • → Obstruction
  8. Organ: Pulmonary
    Abnormality:
    Result:
    • Organ: Pulmonary
    • Abnormality:
    • → Thick (hard) secretions
    • → Infection
    • Result:
    • → Chronic obstruction
    • → Chronic infections
  9. Germs Found in the lungs of CF patients: S. aureus
    • 2-4 years old have a lot
    • Decreased amount in the lungs as they get older
    • Not treated
  10. Germs Found in the lungs of CF patients: P. aeruginosa
    • Must be treated
    • Amount in lungs increases w/age
    • Leads to decreased lung function
  11. Germs Found in the lungs of CF patients: Achromobacter
    • Not treated in the US
    • Treated everywhere else (Europe: France)
  12. Germs Found in the lungs of CF patients: S. maltophilia
    Treated
  13. Germs Found in the lungs of CF patients: Burkholderia cepacia
    • Leads to decreased mortality/morbidity
    • Not allowed to be near other non-cepacia CF patients (be on a different floor, no interaction, completely isolated)
  14. Sinus/Lung Pathophysiology of CF
    • Sinus cavity polyps
    • Shortness of breath
    • Cough
    • Sputum production
    • Barrel chest (air trapping)
    • Decreased FEV1
    • Bacterial growth in the lungs
    • → Exacerbations
    • → Increased cough
    • → Reduction in pulmonary function
    • → Increased sputum production
    •     ¤ Sepsis from a bacterial lung infection
  15. GI Pathophysiology of CF
    • Steatorrhea (greasy stools)
    • Failure to thrive
    • Malnutrition
    • → Fat soluble vitamin deficiency, decreased pancreatic enzyme, fat malabsorption
    • Infants/Small children
    • → Increase frequency of small stools
    • → Meconium ileus (newborns)
    •     ¤ 1st stool passed after birth being blocked
    • Older patients
    • → Constipation
    • → Abdominal cramping/flatulence
  16. Endocrine Pathophysiology of CF
    • Insulin deficiency
    • Weight loss
    • Increase in blood glucose levels
    • Failed oral glucose tolerance test (OGTT)

    • Cystic Fibrosis Related Diabetes (CFRD)
    • → Seen in 20% of adolescents and 50% of adults
    • → Shares features of Type 1 and Type 2
    • → Primarily caused by insulin insufficiency
    • → Fluctuating insulin resistance also seen
    • → Treated with insulin
  17. Reproductive Pathophysiology of CF
    • Azoospermia
    • → Blockage of vas deferens
    • → Absence of vas deferens
    • Females may have lower fertility due to decreased water content of the cervical fluid
  18. Dx of CF
    • All infants receive newborn screening (NBS)
    • → Identifies high levels of immunoreactive trypsinogen (IRT)
    • → If abnormal, DNA test preformed for known mutations

    • Sweat Chloride Test
    • → Pilocarpine administered transdermally to stimulate sweat gland secretion
    • → If sweat chloride ≥60 mmol/L diagnostic for CF
    •   ¤ If positive then you test for every gene mutation
  19. Respiratory Therapy/”Pulmonary Toilet” for CF
    • 1) Albuterol
    • 2) Hypertonic Saline
    • 3) Dornase alfa
    • 4) Airway Clearance
    • 5) Inhaled ABx
  20. Step One for CF
    • Albuterol (ProAir®, Ventolin®, Proventil®)
    • Drug Class: Beta2 Agonist
    • Mechanism of Action
    • → Relaxes bronchial smooth muscle
    • → Opens up airways to allow for better delivery of respiratory medications
    • Dose: 2 puffs prior to therapy (2 – 4 times daily)
    • Adverse Effects: May increase blood pressure/heart rate
  21. Step Two for CF
    • Hypertonic Saline (HyperSal®)
    • Drug Class: Mucolytic
    • Mechanism of Action:
    • → Water distribution
    • → Hydrates airway mucus secretions
    • → Increases overall lung function
    • Dose: 4 mL nebulized 2 – 4 times daily
    • → 3%, 5% (not commercially available), 7%
    • Adverse Effects:
    • → Bronchospasm
    • → Irritation (7% more burning than 3% or 5%)
  22. Step Three for CF
    • Dornase alfa (Pulmozyme®)
    • Drug Class: Mucolytic
    • Mechanism of Action
    • → Cleaves neutrophil DNA found in airway secretions (~10% of mucous)
    • → Reduces mucous viscosity
    • → Improved lung function
    • Dose: 2.5 mg nebulized daily
    • → Can’t be mixed with other products
    • → Must be protected from light and refrigerated
    • → Must be used with specific nebulizers
    • Adverse Effects: Chest pain, cough, fever, voice alteration
  23. Step Four for CF
    • Percussion
    • Loosens mucus so it can be cleared by coughing/huffing
    • Past therapies
    • → Chest Physical Therapy (CPT) and Postural Drainage
    • Current therapies
    • → Oscillating Positive Expiratory Pressure (Oscillating PEP)not ideal (depends on amount of work you put into it)
    •     ¤ Examples: Flutter™, Acapella™, Cornet™
    • → High frequency Chest Wall Oscillation
    •     ¤ Vest vibrates at high frequency- not ideal
    • → Nebulizer with oscillating PEP
  24. Step Five: Inhaled ABx (I) for CF
    • Tobramycin (Tobi®)
    • Drug Class: Aminoglycoside Antibiotic
    • Mechanism of Action:
    • → Binds to 30S and 50S ribosomal subunits of bacterial cell membrane
    • → Improves pulmonary function
    • → Decreases density of P. aeruginosa in sputum
    • Dose: 300 mg nebulization every 12 hours for 28 days followed by 28 days off (combat resistance)
    • → Can’t be mixed, refrigerate, use with specific nebulizers, light protected
    • Adverse Effects: Cough, voice alteration, sputum color change
  25. Step Five: Inhaled ABx (II) for CF
    • Azetreonam (Cayston®)
    • Drug Class: Monobactam Antibiotic
    • Mechanism of Action
    • → Inhibits bacterial cell wall synthesis by binding to penicillin binding proteins (PBPs)
    • → Improves pulmonary function
    • → Decreases density of P. aeruginosa in sputum (reserved for resistance to Tobi)
    • Dose: 75 mg nebulized three times daily for 28 days with 28 days off
    • → Must use with specific nebulizer, do not mix, store in fridge before reconstituted
    • → Severe patients can skip off block or switch b/w Tobi and Cayston every month
    • Adverse Effects: Cough, congestion, wheezing, sore throat, fever
  26. Airway Inflammation for CF
    • Azithromycin (Zithromax®)
    • Drug Class: Macrolide Antibiotic
    • Mechanism of Action
    • → Inhibits protein synthesis, binds to 50s subunit
    • → Inhibits neutrophil migration
    • → Decreases production of pro-inflammatory mediators
    • → Improves lung function
    • Dose: 250 mg – 500 mg on Monday, Wednesday, and Friday
    • Adverse Effects: Diarrhea, nausea, QT prolongation, increased risk for mycoplasma infections
    • → Azithromycin is used to Tx mycoplasma, but mycoplasma will become resistant
  27. Fat Absorption for CF
    • Pancreatic Enzymes (Creon®, Pancreaze®, Zenpep®)
    • Drug Class: Pancreatic enzyme (no product is interchangeable)
    • Mechanism of Action:
    • → Contains lipase, amylase and protease
    • → Break down fats, proteins and starch
    • Dose: 500 units of lipase/kg/meal (500 – 2500 units/kg/meal)
    • → take with all meals and with snacks
    • Adverse Effects: Abdominal pain (dose is too high, need to decrease)
  28. Fat Soluble Vitamins for CF
    • Vitamin A, D, E and K (AquaADEKs®)
    • Drug Class: Vitamins
    • Mechanism of Action
    • → Provides fat soluble vitamins to patients who have decreased absorption
    • → Vitamins enclosed in hydrophilic spheres
    • Typical Dose: 1 softgel twice daily with food
    • → usually need more Vit. D & has been reformulated with more Vit. D
    • Adverse Effects: GI Upset
  29. Gene Modulation (I) for CF
    • Ivacaftor (Kalydeco™)
    • Drug Class: Cystic Fibrosis Transmembrane Conductance Regulator Potentiator
    • Mechanism of Action:
    • → Restores function of the defective CFTR protein
    • → Improves salt and water absorption
    • → Approved for 10 rare mutations: G551D, G551S, G178R, G1244E, G1349D, S549N, S549R, S1251N, S1255P and R117H (flaps don't work- open/close properly)
    • Dose: 150 mg PO every 12 hours with high fat foods
    • → Makes flap work
    • → Can stop all CF meds
    • Adverse Effects: Headache, rash, URI, oropharyngeal pain, congestion, nasopharyngitis
    • Heavy CYP 3A4 inducer, decrease dose if decreased liver function, expensive ($3,000/year)
  30. Gene Modulation (II) for CF
    • Lumacaftor and Ivacaftor (Orkambi®)
    • Drug Class: Cystic Fibrosis Transmembrane Conductance Regulator Potentiator
    • Mechanism of Action:
    • → Increased processing and trafficking of mature protein to the cell surface (lumacaftor)
    • → Ivacaftor MOA on previous slide
    • → Approved for homozygous ΔF508 mutation
    • Dose: 400/250 mg every 12 hours with high fat foods
    • Adverse effects: GI, changes in respiration, chest discomfort, dyspnea, nasopharyngitis
    • → $3,000/year, CYP 3A4 inducer, decreased dose in poor liver function
  31. IV Antibiotics for CF
    • Must be given
    • Recommend to be given as an inpatient versus home health
    • No recommendations on whether to continue inhaled antibiotics on admission
    • → Continue all other home treatments
    • On every admission will treat for pseudomonas infections with double coverage
    • Admissions typically last for multiple weeks
    • Other antibiotics will be based on sputum growth
    • Lung transplant is a possibility
  32. IV Antibiotics for Pseudomonas for CF
    • Piperacillin-Tazobactam (Zosyn®)
    • Ceftazidime (Fortaz®)
    • Cefepime (Maxipime®)
    • Meropenem (Merrem®)
    • Levofloxacin (Levaquin®)
    • Gentamicin (once daily dosing)
    • Tobramycin (once daily dosing)
    • Amikacin (once daily dosing)
    • Colistimethate (Coly-Mycin M®)
    • Patients are rapid metabolizers and may need higher doses than average population
Author:
ebmalonzo
ID:
315853
Card Set:
CF
Updated:
2016-02-14 19:16:10
Tags:
IT CF
Folders:
IT 2
Description:
IT2 (MT3): CF
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